Atypical presentation of atypical amyloid.

Amyloidosis is a group of diseases categorized by precipitation of a group of protein aggregates (amyloid) in tissues, including the kidney, and proteinuria is usually the commonest, though not exclusive, hallmark of clinical presentation. AL and AA are the most commonly recognized forms of amyloidosis involving the kidney, but other forms have been described. We present a case of renal amyloidosis due to a novel amyloidogenic protein, leucocyte cell-derived chemotaxin 2, without proteinuria at presentation or on subsequent follow-up.

Predicting orthopedic involvement in patients with lipomyelomeningoceles.

INTRODUCTION: Lipomyelomeningoceles (LMM) occur in approximately 1 in every 4,000 live births in the United States. They are associated with a wide range of problems in affected patients, including skin abnormalities, sensory and motor deficits, pain, urinary bladder and anal sphincter dysfunction, and orthopedic deformities. MATERIALS AND METHODS: In an effort to better understand the orthopedic complications associated with LMM, the present study examined the long-term orthopedic deformities in 50 patients after surgical correction of their LMM and observed for correlation between these deformities and the type and level of LMM. RESULTS: Analysis of the collected data revealed a statistically significant relationship between of the type of LMM and the presence of orthopedic complications. However, no statistically significant relationship existed between the vertebral level of the LMM and the presence of orthopedic deformities. No correlation was identified between the level and type of LMM. CONCLUSION: In this study, caudal LMM were much more likely than either dorsal or transitional types to have orthopedic complications. These data may prove useful to clinicians in predicting outcome and in counseling patients and their parents.